Bilateral renal hilar tumors in IgG4-related disease

Excerpt

A 51-year-old man visited our hospital due to renal dysfunction. Physical examination revealed bilateral palpebral edema, swelling of the cervical lymph node and salivary gland. Laboratory data and urinalysis revealed the following: creatinine 5.99 mg/dL; IgG 3166 mg/dL; IgG4 1440 mg/dL; uric protein 0.12 g/gCre; and urine beta 2-microglobulin 2401 µg/L. Computed tomography showed bilateral hydronephrosis and renal hilar tumors (Fig. 1a). Renal dysfunction caused by post-renal obstruction was suspected. Double J catheter insertion in bilateral ureters did not improve his renal function completely. Renal biopsy made a diagnosis of tubulointerstitial nephritis by IgG4-related kidney disease (IgG4RKD) (Fig. 2). Oral prednisolone, which was administered at 40 mg and tapered to 10 mg for 6 months, improved his renal function (creatinine 1.69 mg/dL) and reduced bilateral renal hilar tumors (Fig. 1b). …